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Physiological and pathological functions of PQBP-1

Research Project

Project/Area Number 12670596
Research Category

Grant-in-Aid for Scientific Research (C)

Allocation TypeSingle-year Grants
Section一般
Research Field Neurology
Research InstitutionThe University of Tokyo

Principal Investigator

OKAZAWA Hitoshi  Univ. Tokyo, Dept. Neurology, Res. Associate, 医学部・附属病院, 助手 (50261996)

Project Period (FY) 2000 – 2001
Project Status Completed (Fiscal Year 2001)
Budget Amount *help
¥3,300,000 (Direct Cost: ¥3,300,000)
Fiscal Year 2001: ¥1,100,000 (Direct Cost: ¥1,100,000)
Fiscal Year 2000: ¥2,200,000 (Direct Cost: ¥2,200,000)
KeywordsPQBP-1 / polyglutamine / transcription / cell death / neuron / spinocerebellar degeneration / ポリグルタミン / 神経細胞死 / U5-15kD / スプライシング / スプライミング
Research Abstract

In this project, we have analyzed physiological and pathological functions of PQBP-1, which we found as a novel binding protein to the polyglutamine sequences. First, we found that PQBP-1 also interacts with ataxin-1, a causative gene product of SCA1, and induces transcriptional repression cooperatively. The interaction between PQBP-1 and ataxin-1 inhibits transcription not in the inclusion body but in the nuclear matrix, suggesting a new cascade of pathogenesis. Furthermore, our study showed that the interaction leads to reduction of phosphorylated form (=active form) of RNA polymerase II. These results, together with transgenic mouse of PQBP-1, will contribute for understanding this type of neurodegenerative diseases.

Report

(3 results)
  • 2001 Annual Research Report   Final Research Report Summary
  • 2000 Annual Research Report
  • Research Products

    (12 results)

All Other

All Publications (12 results)

  • [Publications] Shoji, M. et al.: "JNK activation is associated with intracellular β-amyloid accumulation"Molecular Brain Research. 85. 221-233 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Okazawa, H. et al.: "PQBP-1 (Np/PQ) : A polyglutamine tract binding and nuclear inclusion-formin protein"Brain Research Bulletin. 56. 273-280 (2001)

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Ueda H, Goto J, Hashida H, Lin X, Oyanagi K, kawano H, et al.: "Enhanced SUMOylation in polyglutamine diseases"Biochem. Biophys. Res. Commun.. (in press).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Okazawa H, Rich T, Chang A, Lin X, Waragai M, et al.: "Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death"Neuron. (in press).

    • Description
      「研究成果報告書概要(和文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Shoji M., Iwakami N., Takeuchi S., Waragai M., Suzuki M., Kanazawa M., Lippa C.F., Ono S. and Okazawa H.: "JNK activation is associated with intracellular beta-amyloid accumulation."Molecular Brain Research. 85. 221-233 (2000)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Okazawa H., Sudol, M. and Rich, T.: "PQBP-1 (Np/PQ): a polyglutamine tract-binding and nuclear inclusion-forming protein."Brain Res Bull.. 56. 273-280 (2001)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Ueda H., Goto J., Hashida H., Lin X., Oyanagi K., Kawano H., Zoghbi H.Y., Kanazawa I., and Okazawa H.: "Enhanced SUMOylation in polyglutamine diseases."Biochem. Biophys. Res. Commun.. (in press). (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Okazawa H.. Rich T., Chang A., Lin X., Waragai M., Kajikawa M., Enokido Y., Komuo A., Kato S., Shibata M., Hatanaka H., Mouradian M.M., Sudol M., Kanazawa I.: "Interaction between mutant ataxin-1 and PQBP-1 affects transcription and cell death."Neuron. (in press). (2002)

    • Description
      「研究成果報告書概要(欧文)」より
    • Related Report
      2001 Final Research Report Summary
  • [Publications] Shoji, M. et al.: "JNK activation is associated with intracellular β-amyloid accumulation"Molecular Brain Research. 85. 221-233 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Okazawa, H. et al.: "PQBP-1(Np/PQ): Apolyglutamine tract binding and nuclear inclusion forming protein"Brain Research Bulletin. 56. 273-280 (2001)

    • Related Report
      2001 Annual Research Report
  • [Publications] Waragai M. et al.: "PQBP-1/Npw38, a nuclear protein binding to the polyglutamine tract, interacts with U5-15KD/dim1p via the Carboxyl-terminal domain."Biochem.Biophys.Res.Commun.. 273. 592-595 (2000)

    • Related Report
      2000 Annual Research Report
  • [Publications] Shoji M. et al.: "JNK activation is associated with intracellular β-amyloid accumulation."Molecular Brain Research. 85. 221-223 (2001)

    • Related Report
      2000 Annual Research Report

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Published: 2000-04-01   Modified: 2016-04-21  

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